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  • Proximal Femoral Focal Deficiency 3

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    chinmayghaisas
    73 vizualizări26 pagini
    This document summarizes proximal femoral focal deficiency (PFFD), a rare birth defect affecting the proximal femur. It discusses the classification systems used to describe PFFD, including those proposed by Aitken, Pappas, Kalamchi, and Ainstutz. Non-operative management includes extension prostheses, while operative options depend on the classification and can include osteotomies, epiphyseal synostoses, amputations, rotation plasties, and limb lengthening procedures like the Ilizarov technique. Treatment is highly individualized and aims to address problems like hip instability, malrotation, muscle deficiency, and limb length discrepancy.

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    This document summarizes proximal femoral focal deficiency (PFFD), a rare birth defect affecting the proximal femur. It discusses the classification systems used to describe PFFD, including those proposed by Aitken, Pappas, Kalamchi, and Ainstutz. Non-operative management includes extension prostheses, while operative options depend on the classification and can include osteotomies, epiphyseal synostoses, amputations, rotation plasties, and limb lengthening procedures like the Ilizarov technique. Treatment is highly individualized and aims to address problems like hip instability, malrotation, muscle deficiency, and limb length discrepancy.

    Drepturi de autor:

    © All Rights Reserved
    Descărcați ca PPTX, PDF, TXT sau citiți online pe Scribd
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    73 vizualizări26 pagini

    Proximal Femoral Focal Deficiency 3

    Încărcat de

    chinmayghaisas
    This document summarizes proximal femoral focal deficiency (PFFD), a rare birth defect affecting the proximal femur. It discusses the classification systems used to describe PFFD, including those proposed by Aitken, Pappas, Kalamchi, and Ainstutz. Non-operative management includes extension prostheses, while operative options depend on the classification and can include osteotomies, epiphyseal synostoses, amputations, rotation plasties, and limb lengthening procedures like the Ilizarov technique. Treatment is highly individualized and aims to address problems like hip instability, malrotation, muscle deficiency, and limb length discrepancy.

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    din 26

    BRIEF DISSCUSION ON PROXIMAL

    FEMORAL FOCAL DEFICIENCY

    MODERATORS: DR PREM KOTIAN


    DR K R KAMATH
    PRESENTER : DR NIKIL JAYASHEELAN
    DATE: 16thth FEBRUARY 2010

    Broad spectrum ofdefects


    Most commonly- partial skeletal

    defect in the proximal femur


    with a variably unstable hip.
    Shortening, and associated other
    anomalies.
    Incidence of - 1 per 50,000 live
    births

    Fibular hemimelia
    Clubfoot
    Spinal dysplasia
    Facial dysplasia
    Agenesis of the cruciate

    ligaments of the knee.

    ETIOLOGY
    Maternal ingestion of drugs e.g: Thalidomide
    Deficit of

    bone-cartilage , scleroterm subtraction

    Disturbances in cellular nutrition


    Vascular abnormality

    HISTOLOGICAL
    Abnormal proliferation of chondrocytes
    Abnormal maturation of chondrocytes in proximal

    growth zone
    Disorganized matrix- inadequate preparation of matrix-

    delay vascular invasion


    cartilaginous necrosis enlarged hypertrophic zone,

    CLASSIFICATION
    1.

    Aitken's

    2.

    Pappas

    3.

    Kalamchi et al

    4.

    Ainstutz

    Aitken's four-part classification

    Normal acetabulum and


    femoral head
    shortening of the femur

    No bony connection between the


    proximal femur and the femoral
    head,
    Pseudoarthosis

    Aitken's four-part classification

    Dysplastic acetabulum,
    Absent femoral head,
    Short femur.

    Acetabulum, femoral head,


    and proximal femur are
    totally absent

    PAPPAS NINE CLASSES

    KALAMCHI ET AL FIVE GROUPS


    Group I- short femur and intact hip joint
    Group II- short femur and coxa vara of the hip
    Group III- short femur but well-developed acetabulum

    and femoral head


    Group IV- absent hip joint and dysplastic femoral

    segment
    Group V- total absence of the femur.

    Ainstutz (1969)
    Type 1. Congenital short femur with bowing,
    coxa vara and normal acetabulum

    Type 2. Short femur with subtrochanteric


    pseudarthrosis, progressive coxa vara and
    normal acetabulum.

    Type 3 Short femur with a bulbous proximal end,


    delayed appearance of the femoral capital
    epiphysis. Acetabulum is mildly dysplastic

    Type 4. Short femoral segment tapering


    sharply to a point at proximal end.
    Acetabulum more dysplastic

    Type 5 . Small bony segment representing distal


    femoral shaft, with no evidence of proximal
    femoral components and no acetabulum.

    MANAGEMENT
    Basic problems
    1.
    2.
    3.
    4.

    Instability of the hip


    Malrotation
    Inadequate proximal musculature
    Inequality of leg length

    Treatment
    Bilateral PFFD is best treated non-operatively
    Treatment is highly individualized -ranges from

    amputation and prosthetic rehabilitation to limb salvage,


    lengthening, & hip reconstruction.
    surgery is best delayed until ossification of the femoral

    head and proximal metaphysis is adequate.

    Non- Operative Treatment


    Extension prosthesis

    Operative Treatment
    Hip Stability

    Class-A : osteotomy at the site of the pseudarthrosis.


    Class-B : Metaphyseal epiphyseal synostosis .

    subtrochanteric osteotomy

    Amputations

    Above-the-Knee Amputation
    Below knee amputation

    ROTATION PLASTY
    Described byVan Nes ,
    Modified by Kostuik et al.& later by Gillespie & Torode
    Arthrodesis of

    knee with rotation of distal tibia 180


    degrees externally so that the ankle joint becomes a
    functional knee joint
    ankle plantar flexion becomes knee" extension and
    ankle dorsiflexion becomes "knee" flexion

    Van Nes rotation-plasty

    Limb lenghtening procedures


    Ilizarov
    Bone transport

    Ilizarov

    Knee arthrodesis

    THANK YOU

    Thank you

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